Teaching NeuroImages: Nonfluent variant primary progressive aphasia

A 66-year-old woman presented with 4 years of progressive speech difficulty. She had nonfluent speech with phonemic errors but intact single-word comprehension and object knowledge. Her grammar was impaired in both speech and writing, and she exhibited orofacial apraxia. A clinico-radiologic (see figure) diagnosis of nonfluent variant primary progressive aphasia was made. Nonfluent variant primary progressive aphasia is a neurodegenerative disease within the spectrum of frontotemporal dementia, characterized by the typical language and brain atrophy patterns seen here. It is most frequently due to tau pathology, and clinicians should be alert to the potential development of progressive supranuclear palsy or corticobasal syndrome.